ueg education Mistakes in… 2020
Mistakes in pouchitis and how to avoid them
Jonathan P Segal, Susan K Clark and Ailsa L Hart
Restorative proctocolectomy can be considered a quality-of-life surgical procedure for patients who have ulcerative colitis that has not responded to medical therapy, and for some patients who have familial adenomatous polyposis. The procedure removes the entire diseased large bowel and utilises the patient’s small bowel to create a reservoir that allows defaecation without the need for a long-term ileostomy. Despite generally good outcomes, complications can occur. One of the most frequent problems is primary idiopathic pouchitis, which is characterized by increased stool frequency, haematochezia, abdominal cramping, urgency, tenesmus, incontinence, fever and flare-up of extraintestinal manifestations.1 The incidence of acute primary idiopathic pouchitis following surgery has been reported to be 20% at 1 year, and up to 40% at 5 years.2 Chronic primary idiopathic pouchitis develops in 10–15% of all patients who undergo restorative proctocolectomy for ulcerative colitis and can be ‘responsive’ or ‘refractory’ to antibiotic therapy. Chronic pouchitis is defined as symptoms of pouchitis that persist beyond 4 weeks or multiple relapses of acute pouchitis within a year.3,4 Here we discuss mistakes in the assessment and management of primary idiopathic pouchitis and how best to avoid them. Most of the discussion is evidenced based, but where evi- dence is lacking the discussion is based on our extensive clinical experience of treating patients who have pouch dysfunction.
Mistake 1 | Assuming all pouch dysfunction Campylobacter, Salmonella, Candida or pelvic sepsis on MRI.7 It is therefore imperative to is primary idiopathic pouchitis without cytomegalovirus), pelvic sepsis, faecal stasis, thoroughly assess a dysfunctional pouch with MRI. excluding alternative diagnoses ischaemia or drugs (particularly NSAIDs).8 The potential consequences of missing this When patients present with symptoms related diagnosis are inappropriate use of antibiotics, There are no validated scoring systems available to their pouch it is important to be mindful that antibiotic resistance, side effects and worsening to define pouchitis. The pouch disease activity other possible causes (e.g. hyperthyroidism, clinical state. Furthermore, by missing pelvic index (PDAI)5 is used in the research setting but it coeliac disease or pouch cancer) are excluded. sepsis and assuming symptoms are primary remains unvalidated and its routine use in clinical This is best done by following an algorithm, such idopathic pouchitis, there is the potential practice is rare. as the one presented in figure 1.9 to escalate to biologics, which may cause Importantly, there can be a lack of correlation It is essential to take a robust history, perform immunosuppression and worsening sepsis. between symptoms, endoscopic activity a thorough examination and offer timely Should pelvic sepsis be found, appropriate and histological findings, leading to potential investigations (including pouchoscopy and antibiotic therapy should be considered, with a misdiagnosis.6,7 Furthermore, there is a radiological examination) before confirming a multidisciplinary discussion to plan management tendency to label every patient who has pouch diagnosis of primary idiopathic pouchitis. An that may include drainage and diversion. dysfunction as having pouchitis; primary important question to ask is “Has the pouch ever idiopathic pouchitis is often diagnosed without worked well?” If this is not the case, a diagnosis Mistake 3 | Not systematically assessing endoscopic and histological confirmation, which of anastomotic leak/pelvic sepsis needs and reporting all pouch regions can result in other pathologies being missed. In careful consideration. Importantly, it is essential endoscopically addition, patients often self-diagnose primary to establish the baseline pouch function for an idiopathic pouchitis based on their symptoms, individual patient; the median 24-hour stool Thorough endoscopic assessment is vital to without objective markers of inflammation or frequency is four to eight, with roughly half of help understand the potential reasons for a endoscopic assessment being used. patients needing to defecate at night. poorly functional pouch. However, no validated It is also important to distinguish between endoscopic reporting systems, key performance primary and secondary types of pouchitis. Mistake 2 | Missing pelvic sepsis as a cause indicators, training or certification are available to Primary idiopathic pouchitis is defined as of pouchitis assess competency in pouchoscopy. Furthermore, pouchitis when all secondary causes have been there is considerable variation in the quality of excluded, whereas secondary pouchitis can In one retrospective study, more than a third of endoscopy reports and efforts have only recently be due to Crohn’s disease, infection (e.g. with patients considered to have antibiotic-dependent been made to develop a reporting template Clostridiodes [formerly Clostridium] difficile, pouchitis were actually found to have evidence of specific to pouchoscopy.10 The standardised
© (2020) Segal, Clark and Hart. Surgery and Cancer, Imperial College London, United Kingdom. Correspondence to: [email protected] Cite this article as: Segal JP, Clark SK and Hart AL. Mistakes in Ailsa Hart is a consultant gastroenterologist at St Mark’s Hospital, Conflict of interest: The authors declare they have no conflicts of pouchitis and how to avoid them. UEG Education 2020; 20: 7–11. Harrow, United Kingdom and in the Faculty of Medicine, interest to declare in relation to this article. Jonathan Segal is a specialist gastreonterology registrar and Department of Metabolism, Digestion and Reproduction, Imperial Published online: July 9, 2020. Susan Clark is a consultant colorectal surgeon at St Mark’s College London, United Kingdom. Hospital, Harrow, United Kingdom and in the Department of Illustrations: J. Shadwell.
7 ueg education Mistakes in… 2020 reporting template introduced is a systematic tool respond to medications such as tricyclic poor outcomes and such surgery is done highly that prompts examination of the anal/perineal antidepressants. selectively. region, rectal cuff, anastomosis, lower and upper There are essentially two scenarios that pouch body, pouch inlet and pre-pouch ileum, but Mistake 5 | Not recognising the presence lead to a diagnosis of ‘Crohn’s disease of the it is not widely used. of cuffitis pouch’. The first arises when there has been a Other problems with pouchoscopy include preoperative diagnosis of Crohn’s disease, and poor intra- and inter-rater reliability between When forming a pouch, there is an anastomosis the second is the development of Crohn’s-like pouchitis scoring systems,11 with some of the between the ileal pouch and the anorectum. features after the formation of a pouch in a PDAI descriptors considered inappropriate for In some situations, this area—refered to as the patient who has ulcerative colitis. assessing endoscopic disease activity in cuff—can become inflamed, resulting in ‘cuffitis’. In one long-term study, 2–8% of patients pouchitis. This suggests that we need better Essentially this is residual ulcerative proctitis. who originally underwent restorative validated scoring systems for assessment of The symptoms of cuffitis are characterised by the proctocolectomy for presumed ulcerative colitis pouch inflammation.11 frequent passage of stool with small quantities of had their original diagnosis changed to Crohn’s We suggest that endoscopic examination of a blood,13 urgency and pain and it can, therefore, be disease.21 We believe it is important to diagnose pouch is performed by experienced endoscopists mistaken for pouchitis. Crohn’s disease of the pouch accurately to help and that a systematic template should be used to A digital examination should be performed to with prognostication and offer appropriate encourage examination (and biopsy) of all pouch manually feel the cuff. A long retained rectal cuff treatment. The criteria utilised to diagnose regions and recording of findings. Photographic (longer than 2cm) is more prone to inflammation. Crohn’s disease are varied. Some studies have evidence of the pouchoscopy can also help Endoscopic evaluation is required to directly defined Crohn’s disease of the pouch as assessment. visualise the cuff, with biopsy samples taken including: inflammation of the pouch that is for histological assessment to confirm the resistant to antibiotic treatment, stricturing of the Mistake 4 | Failing to appreciate the impact diagnosis.13–15 The incidence of inflammation afferent limb, stricturing of the small bowel, or of symptoms on quality of life of the retained anorectum has not been fistulating disease.27–30 Furthermore, the presence extensively studied; some studies report a 9% of pre-pouch ileitis is controversial—some There is a large variation in how different patients incidence.16 studies suggest this may be an endoscopic perceive their pouch symptoms and how their The treatment for cuffitis differs from that feature of Crohn’s disease,31,32 but this has been symptoms impact on their overall quality of life for pouchitis and so distinguishing the two disputed by others.33 (QoL). There can also be a disconnect between is key. Although cuffitis is a poorly studied It is likely that Crohn’s disease of the symptom burden and the severity of inflammation condition, there is evidence that mesalamine pouch is an overused diagnosis, with one study noted in the pouch. Indeed, some patients with suppositories14 may provide some benefit, with highlighting that histological confirmation of very minimal objective inflammation noted steroid suppositories as a second-line therapy.17 Crohn’s disease was found in only 20% of patients endoscopically and histologically can have pouch Essentially the management of cuffitis is the who underwent pouch excision for Crohn’s function that severely impacts on their quality of same as treating ulcerative proctitis. disease of the pouch.33 It is also important to life, whereas other patients can report minimal appreciate that strictures and fistulas that may symptoms but have severe inflammation noted. Mistake 6 | Labelling the diagnosis as mimic Crohn’s disease can be caused by It is essential to be conscious of this variability Crohn’s disease when the features may be other factors such as sepsis, anastomotic and discrepancy, so that investigation and due to another aetiology complications (e.g. leak and/or stricture) or management is tailored to the individual patient. ischaemia. We therefore suggest that Crohn’s In patients who have a high symptom burden Prior to pouch formation a thorough pre-surgical disease of the pouch should only be diagnosed but little inflammation, other causes of their assessment must be considered to rule out by conclusive histology (i.e. granulomas symptoms should be explored along with all Crohn’s disease, to include small-bowel studies, supporting Crohn’s disease) and/or the presence supportive options available, including histological samples reviewed for the presence of characteristic skip lesions in the small bowel. psychological support. of granuloma and the absence of a history of The timing of when the Crohn’s-like problems of The clinical scoring systems do not take into perianal disease. A preoperative diagnosis of the pouch occur can often aid diagnosis—fistulas account QoL indicators that may be significant to Crohn’s disease of the pouch is a relative especially around the anastomosis that occur a patient, which means it is vital to consider contraindication to pouch surgery18 because of the within a year of pouch formation are likely to a patient’s quality of life when assessing high rate of complications and pouch be related to the surgery itself, whereas symptomatology. We recommend that a broad failure.19–21 Indeed, it has been estimated that complications beyond this point could represent range of domains (including effect on personal pouch excision rates are 45–55% in patients de novo Crohn’s disease, but this still requires life, work, sexuality and overall quality of life) who have a preoperative diagnosis of Crohn’s thorough investigation. are taken into account. To aid such assessment, disease21,22 and that the pouch retention rate We also believe that it can be unhelpful and a multidisciplinary team of pouch and stoma 5 and 10 years after formation is 58% and 50%, confusing for patients to hear that their diagnosis nurses, psychologists, clinicians and patient-to- respectively.23 Despite this, some small studies has changed from ulcerative colitis to Crohn’s patient support can be valuable when helping have shown that in the absence of perianal or disease and suggest it is more useful to patients with pouch-related issues. small-bowel disease, restorative proctocolectomy explain that they have active inflammatory Specifically, it is important to assess the can be performed with similar outcomes to those bowel disease. It is more beneficial for the mental health of a patient who has poor pouch who have a pouch for ulcerative colitis.24,25 Indeed, patient to be clear on the strategies of care for function and offer them psychological support. the European Crohn’s and Colitis Organisation managing their problem, whether it be a There has been evidence to show that adjuncts (ECCO) suggest that restorative proctocolectomy fistula, stricture or inflammation, using the such as biofeedback may be beneficial.12 Irritable can be offered to patients who have Crohn’s appropriate combination of surgical/endoscopic pouch syndrome (analogous to irritable bowel disease without perianal disease or small-bowel and medical therapies, than to become syndrome) is diagnosed when no other pathology involvement,26 but we suggest that patients are bogged down in semantics regarding is present to explain symptoms, and may very carefully counselled about the potential terminology.
8 ueg education Mistakes in… 2020
cautiously, especially in the elderly.37 We as t e ouc e e o ked e e advise regular reassessment of patients on ist r at is t e ti ing o inc eased ouc s to s in e ation to su ge long-term antibiotics, including QoL assessment, uesti ns o an e ious s to atic e isodes a e ou ad sing e e sus ecu ent e sus ongoing endoscopic and radiological investigations and o a e ou s to s e ious es onded to anti iotics consideration of alternative medications where at is ou ast isto o gast ointestina in ections and at appropriate. As already described, more is ou t a e isto than a third of patients considered to have at i an s ste ic s to s do ou a e antibiotic-dependent pouchitis were found to at i an e t aintestina s to s do ou a e skin e e oints have evidence of pelvic sepsis on MRI7 and hence • Do you experience difficult evacuation? at edications do ou take inc uding s this should be excluded before a diagnosis of chronic antibiotic-dependent primary idiopathic pouchitis is made.